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Diagnosed with pulmonary arterial hypertension. Took oxygen therapy. problem persists. Further treatment?

This is the case related to Pulmonary Arterial Hypertension. She has been suffering from a chronic disease called Systemic Sclerosis since 1998 and something called Interstitial Lung Disease since 2000. She has been under regular medications and checkups at AIMS, New Delhi and her condition was quite stable till the year end 2011. However, suddenly since November 2011, she started feeling the problem of acute cough , shortness of breath, for which she was treated with inhalers and nebulisers in Central Hospital, Dhanbad during December-January, 2012. However, her condition further deteriorated and she went to Apollo, Delhi in February and was diagnosed with Pulmonary Arterial Hypertension. The medications which they gave her did not suit her body and her condition was further very bad, Finally, she got admitted in CMRI, Kolkata Hospital in March for a month and there her condition became under control and they advised her with medicines and Long term Oxygen Therapy (she has to take oxygen for around 18 hours, which they said will reduce as the medicines start acting). And yes, they said that Pulmonary Hypertension is once again a chronic incurable disease. She accepted that fact as well, was under oxygen and bed rest for another month and She was quite stable in the month of June and was taking oxygen for 15-16 hours per day and she started going to my department for around 4 hours and also started taking a few lectures in the month of August-October,2012....with of course a few up and downs in her health every ten days. She was determined to fight it out and was contend with this life as well. But suddenly, again since the end of October, her lung condition started deteriorating and she was not able to get up or breath properly. She was finally again admitted at CMRI, Kolkata on November, 15 and this time She was very ill and she had to be under oxygen for almost 24 hours. They changed some medications and kept her there for 16 days. She was discharged from there on November 30 and they said that after taking rest and new medicines...hopefully my condition would improve in a month s time. So, we are looking for any medication, if available. If required, I can send her medical records. Awaiting your help in this regard.
Asked On : Sat, 22 Dec 2012
Answers:  1 Views:  103
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Pulmonologist 's  Response
The patient seems to be suffering from systemic sclerosis associated interstitial lung disease with pulmonary artery hypertension. At the outset , I would like to mention that this will be an irreversible condition. Long term oxygen therapy helps to improve the quality of life and assists the lungs in the process of oxygenation and also helps the pulmonary artery hypertension which seems to have been resulted from hypoxemic vasoconstriction. Apart from medicines one of the important measures is avoiding cold exposures. Treatment consists of inhaled medicines, oral steroids or immunosppresants as and when required. Medications also depend on the extent of other organ involvement of the disease like kidney or skin (does skin changes to blue in cold?). Some supportive managements include yearly flu vaccination and 5 yearly pneumococcal vaccination. Do not use nebuliser daily , instead use metered dose inhalers with spacer. Monitor the pulmonary function tests regularly in consultation with a pulmonologist. Medications like abrixentan or taldalafil may be of some help for the pulmonary artery hypertension but need to be taken under supervision of a pulomonologist. If the lung involvement is extensive then lung / heart lung transplant can be done in selected patients on individual basis.


Dr. Gyanshankar Mishra
Consultant Pulmonologist
Answered: Mon, 24 Dec 2012
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